" /> Mikulicz disease - CISMeF





ICD-11 code : 4A43.1;

Preferred Label : Mikulicz disease;

ICD-11 definition : Mikulicz disease is a disorder first reported by Johann von Mikulicz in 1892 and characterized by symmetrical swelling of the lachrymal, submandibular, and parotid glands, with massive infiltration of these glands by mononuclear cells. Serum autoantibodies, such as anti-Ro/SS-A, are usually negative and serum IgG4 concentration may be increased. Unlike Sjögren disease, IgG4-Mikulicz disease is characterized by the formation of lymphoid follicles, but shows lower levels of lymphocytic infiltration into salivary ducts, such that their structure remains intact.;

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Mikulicz disease is a disorder first reported by Johann von Mikulicz in 1892 and characterized by symmetrical swelling of the lachrymal, submandibular, and parotid glands, with massive infiltration of these glands by mononuclear cells. Serum autoantibodies, such as anti-Ro/SS-A, are usually negative and serum IgG4 concentration may be increased. Unlike Sjögren disease, IgG4-Mikulicz disease is characterized by the formation of lymphoid follicles, but shows lower levels of lymphocytic infiltration into salivary ducts, such that their structure remains intact.

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30/07/2025


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